Stewart-Bluefarb syndrome is a rare angioproliferative disorder characterised by acroangiodermatitis associated with an underlying arteriovenous shunt. This condition should be differentiated from acroangiodermatitis of Mali classically described in association with chronic venous insufficiency. Patients with Stewart-Bluefarb syndrome typically present with lower leg pigmented macules, papules and plaques that can coalesce ...

Scimitar syndrome is a congenital heart disease characterized by an abnormal drainage of the right lung into the inferior vena cava, the right atrium or a variety of venous connections from the anomalous pulmonary vein to a systemic vein. This left-to-right shunt induces pulmonary hypertension and is an indication for ...

Although thrombosis is considered the cardinal feature of the antiphospholipid syndrome, chronic vascular lesions are common, particularly in patients with life-threatening complications. In patients who require transplantation, vascular lesions often recur. The molecular pathways involved in the vasculopathy of the antiphospholipid syndrome are unknown, and adequate therapies are lacking. We ...

Post-thrombotic syndrome is a chronic complication of acute deep venous thrombosis in the lower extremity. The role of graduated compressive stockings in the prevention of post-thrombotic syndrome has been studied with opinion being divided on the beneficial effects. We aim to answer this question with a randomized controlled study that ...

Acute coronary syndromes continue to be a dominant cause of death worldwide. Acute therapies with potent anti-thrombotic agents have improved in-hospital outcomes, however, despite the use of these therapies, recurrent events continue to occur long-term. Statins, renin-angiotensin inhibitors, beta-blockers and dual anti-platelet therapy all have roles in mitigating long-term risk, ...

Patients with acute coronary syndrome (ACS) are typically managed with long-term dual antiplatelet therapy of acetylsalicylic acid plus a P2Y12 platelet receptor antagonist; however, although effective, the risk of another vascular event within 12 months remains at approximately 10%. Considerable efforts have been made to find improved therapeutic approaches to ...

Abstract The aim of this review is to analyse the pathophysiology and complications of thrombosis in conjuction with ovarian hyperstimulation syndrome (OHSS) following ovulation induction and to suggest practical guidelines usefull for the prevention and treatment. Although the incidence of thrombosis varies from 0.2% among in vitro fertilization (IVF) cycles ...

May-Thurner syndrome is a rare clinical entity involving venous obstruction of the left lower extremity. The May-Thurner syndrome is a phenomenon commonly described as an acquired stenosis of the left common iliac vein secondary to compression of the left common iliac vein between the right common iliac artery and the ...

A 36-year-old woman presented with right shoulder weakness following a left parotid tumor resection. The overall clinical presentation included severe paralysis and atrophy of the right sternocleidomastoid and upper trapezius, an absent right gag reflex, and diminished right posterior tongue pinprick sensation. A diagnosis of right-sided Vernet syndrome (cranial nerve ...

Stewart-Bluefarb acroangiodermatitis is the occurrence of pseudo-kaposiform changes with an underlying arterio-venous malformation. Parkes-Weber syndrome is a triad of arterio-venous malformation with varicose veins with bony and soft-tissue hypertrophy involving the extremity. A 13 year-old-female presented with ulcerated growth with bleeding episodes on right leg since past four years. A ...

Antiphospholipid syndrome is an autoimmune disorder characterized by arterial or venous thrombosis, recurrent first-trimester pregnancy loss, and multiple additional clinical manifestations. We describe a man with severe atherosclerotic basilar artery stenosis and superimposed in situ thrombus who was found to have antiphospholipid syndrome.

One of the least recognized risks for the development of deep venous thrombosis (DVT) is iliac vein compression or the May-Thurner Syndrome (MTS), in which most often, the right common iliac artery compresses the subjacent left common iliac vein. We present three patients with MTS complicated by massive left lower ...

Neurofibromatosis type 1 (NF1) is the most prevalent autosomal dominant genetic disorder among humans. NF1 vasculopathy is a significant but underrecognized complication of the disease, affecting both arterial and venous blood vessels of all sizes. Moyamoya syndrome is a cerebral vasculopathy that is only rarely observed in association with NF1, ...

Fusobacterium nucleatum is a gram-negative bacillius commonly found in oropharynx and is traditionally associated with Lemierre syndrome, which is characterized by history of recent oropharyngeal infection, internal jugular vein thrombosis, and isolation of anaerobic pathogens, mainly Fuosobacterium necrophorum. However, recent evidence indicated that F. nucleatum is also a normal resident ...

Hereditary hemorrhagic telangiectasia (HHT) and hepatopulmonary syndrome are disorders characterized by the development of multiple pulmonary arteriovenous malformations (PAVM). COX2 may be at the origin of a cascade of pro inflammatory events to favour angiogenesis and PAVM development. HHT and hepatopulmonary syndrome mouse models may be used to show its ...

Venous malformations (VMs) are the most common vascular developmental anomalies (birth defects). These defects are caused by developmental arrest of the venous system during various stages of embryogenesis. VMs remain a difficult diagnostic and therapeutic challenge due to the wide range of clinical presentations, unpredictable clinical course, erratic response to ...

The past 2 decades have witnessed the introduction and demise of several different antithrombotic medications for acute coronary syndromes. Part of the assessment of these compounds has been their effect on thrombotic events relative to the degree of increase in bleeding events. This review will outline the data supporting various ...

Some features of Behçet's syndrome (BS) tend to go together. We aimed to explore the association and timing of various vascular events in both the venous and the arterial vascular tree. We conducted a chart survey on the type and time of vascular involvement of BS. The cross-relationships of involvement ...

Peripheral extracorporeal membrane oxygenation (ECMO) is associated with a not negligible rate of vascular morbidity. Most vascular complications are related to limb ischaemia mainly due to insufficient limb perfusion or embolic events. To the best of our knowledge, this is the first report of a severe epidermolysis and overflow syndrome ...

Katsetos Christos D CD Department of Pediatrics, Drexel University College of Medicine, St. Christopher׳s Hospital for Children, Philadelphia, PA; Department of Pathology and Laboratory Medicine, Drexel University College of Medicine, St. Christopher׳s Hospital for Children, Philadelphia, PA; Department of Neurology, Drexel University College of Medicine, Philadelphia, PA. Electronic address: - - 2014

This case study describes an instance of death in an early term female newborn with congenital apnea in the clinical setting of multiple congenital anomalies (retrognathia, posteriorly rotated ears, camptodactyly, and arthrogryposis) and prenatal history of polyhydramnios. Postmortem neuropathologic findings were significant for tegmental necrosis in the caudal pons and ...

Acute promyelocytic leukemia (APL) has become the most curable form of acute myeloid leukemia after the advent of all-trans retinoic acid (ATRA). However, early deaths (ED) mostly due to the disease-associated coagulopathy remain the major cause of treatment failure. In particular, hemorrhagic events account for 40-65% of ED and several ...

May-Thurner syndrome is a common anatomic variant associated with deep venous thrombosis. There are few reports describing implications for and management in pregnancy. We performed a retrospective review and description of 4 women with May-Thurner syndrome receiving complete or consultative obstetrical care in our practice. Presentations included (1) previous stroke ...

During pregnancy, venous thrombosis of the distal extremities is not uncommon. However, thrombosis in the upper part of the body, such as jugular vein thrombosis, is rare. If underlying causes such as ovarian hyperstimulation syndrome (OHSS) or septic thrombophlebitis (Lemierre's syndrome) are excluded, a serous borderline ovary tumour (BOT) must ...

Spontaneous bleeding resulting in compartment syndrome at the lower adult leg due to acquired hemophilia A is rare. There are no reports on operative management of this entity. We present a case of atraumatic compartment syndrome of the lower leg due to acquired factor VIII deficiency, in an 83-year-old Caucasian ...

Central venous catheters are the most frequent causes of benign central vein stenosis. We report the case of a 79-year-old woman on hemodialysis through a twin catheter in the right internal jugular vein, presenting with superior vena cava (SVC) syndrome with patent SVC. The clinically driven endovascular therapy was conducted ...

We present a woman with severe symptomatic recurrent varicose veins who was treated with endovenous laser ablation and transluminal occlusion of perforator with attempted phlebectomies for extensive varices. The phlebectomies turned out to be near impossible due to friability of the veins. Her treatment was completed with post-operative ultrasound guided ...

The Klippel-Trenaunay syndrome (KTS) is a congenital disorder characterized by capillary malformation, varicosities and bony and soft tissue hypertrophy. This disease has several morbidities like bleeding, deep venous thrombosis, embolic complications and in some cases enlargement of limb that may require amputation. Vascular malformations are segmented and never cross midline. ...

We describe a case of nephrotic syndrome that manifested as a retropharyngeal pseudoabscess. The patient was a 32-year-old man who presented with a short history of throat discomfort and a choking sensation. Laryngoscopy identified bulging of the posterior pharyngeal wall that partially occluded the laryngeal inlet. A lateral neck x-ray ...

Pseudoamniotic band syndrome is a rare iatrogenic complication of twin-to-twin transfusion syndrome treated with foetoscopic procedures. We report a severe pseudoamniotic band syndrome in the recipient monochorionic diamniotic twin pregnancy with twin-to-twin transfusion syndrome following a selective foeticide procedure. A male newborn with a severe circumferential amniotic band in the ...

Kim Ji Soo JS Department of Pediatrics, The Catholic University of Korea College of Medicine, Seoul, - - 2014

Lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS), a very rare disease that is caused by the presence of antifactor II antibodies, is usually counterbalanced by the prothrombotic effect of lupus anticoagulant (LAC). Patients with LAHPS are treated using fresh frozen plasma, steroids, immunosuppressive agents, and immunoglobulins for managing the disease and controlling hemorrhages. ...

Thrombosis is a rare cause of superior vena cava (SVC) syndrome. We report a 37-year-old man hospitalized because of swelling of the face and neck. A computed tomography angiography showed a thrombotic obstruction of SVC. The patient was treated by percutaneous transluminal balloon angioplasty of the SVC and placement of ...

Hemophagocytic lymphohistiocytosis (HLH) is a rare and often fatal hyperinflammatory syndrome characterized by fever, cytopenia, dramatically increased ferritin and hepatosplenomegaly. Here, we describe a previously healthy 39 year old pregnant woman in 30th week of her pregnancy with diarrhoea, intermittent gastrointestinal bleeding and fever of unknown focus. After cesarean section ...

Consideration of the chronology of advances in medical knowledge can provide useful insights into the pathogenesis, diagnosis and treatment of diseases. The antiphospholipid syndrome is an enigmatic disorder and this is reinforced by the misleading associated terminology, the adoption of which results directly from early discoveries relating to the condition. ...

Intentional interruption of the inferior vena cava with caval ligation and a Mobin-Uddin filter was once commonly used to prevent recurrent pulmonary emboli and was associated with significant mortality and morbidity, including a high incidence of post-thrombotic syndrome. Recanalization of an intentionally interrupted inferior vena cava has been rarely described ...

There are some risk factors being more vulnerable to Lemierre's syndrome such as a hypercoagulable state. We report a rare case of Lemierre's syndrome with ethmoid and maxillary sinusitis, bilateral mastoiditis, and sigmoid sinus thrombosis. Genetic study revealed a double heterozygote status in the methylenetetrahydrofolate reductase gene including C677T and ...

A 62-year-old woman presented with stabbing pain over her left temple radiating to her left cheek when bending forwards or coughing. Neurological examination was normal. There were many cutaneous venous prominences over her body. CT and MR brain scans showed multiple venous anomalies and venous occlusive disease of the left ...

Venous thoracic outlet syndrome is a relatively rare condition. One variety that is related to effort, often referred to as Paget-Schroetter syndrome, is seen more often in persons who are athletic or occupationally active. This condition can have significant morbidity if left untreated. Diagnosis is best undertaken with duplex ultrasound ...

The nutcracker syndrome is a rare clinical manifestation of symptoms caused by the compression of the left renal vein by an overriding superior mesenteric artery, an anatomical variant otherwise known as the nutcracker phenomenon. Usually present in women and children, when symptomatic, it commonly presents with hematuria, proteinuria, and chronic ...

We present a 16-year-old male with severe acute respiratory and renal failure as a result of Goodpasture syndrome, requiring venovenous extracorporeal membrane oxygenation (VV-ECMO) for pulmonary haemorrhage. The patient received no systemic anticoagulation for 25 of 26 ECMO days (20 days consecutively) and suffered no coagulation-related adverse events. The patient ...

One of the challenges of managing patients with antiphospholipid syndrome is the prevention of rethrombosis (secondary prophylaxis). Risk stratification, i.e. traditional cardiovascular and thrombosis risk factors, systemic autoimmune diseases, antiphospholipid antibody profile, and the intensity of anticoagulation are all relevant to the management of APS patients with recurrent thrombosis. The ...

Venous malformations (VMs) are the most common vascular developmental anomalies (birth defects) . These defects are caused by developmental arrest of the venous system during various stages of embryogenesis. VMs remain a difficult diagnostic and therapeutic challenge due to the wide range of clinical presentations, unpredictable clinical course, erratic response ...

The association between aortic stenosis and gastrointenstinal bleeding due to acquired von Willebrand syndrome (AVWS) has been well established. We report a patient with AVWS from underlying severe endocarditis-associated aortic regurgitation and pre-existing mitral regurgitation. Cardiovascular disease-related AVWS occurs from a selective loss of the largest multimers of von Willebrand ...

Abstract It has long been recognized that patients with myositis and positive anti-Jo1 antibody tend to be associated with interstitial lung disease. Recent studies revealed that such patients may also have fever, Raynaud's phenomenon, mechanic's hand, polyarthralgia, or usually mild, self-limiting, non-erosive or erosive polyarthritis known as antisynthetase syndrome. The ...